Year: 2009
Type: article
Abstract: BACKGROUND: The randomized placebo-controlled IFIGENIA-trial demonstrated that therapy with high-dose N-acetylcysteine (NAC) given for one year, added to prednisone and azathioprine, significantly ameliorates (i.e. slows down) disease progression in terms of vital capacity (VC) (+9%) and diffusing capacity (DLco) (+24%) in idiopathic pulmonary fibrosis (IPF). To better understand the clinical implications of these findings we performed additional, explorative analyses of the IFGENIA data set. METHODS: We analysed effects of NAC on VC, DLco, a composite physiologic index (CPI), and mortality in the 155 study-patients. RESULTS: In trial completers the functional indices did not change significantly with NAC, whereas most indices deteriorated with placebo; in non-completers the majority of indices worsened but decline was generally less pronounced in most indices with NAC than with placebo. Most categorical analyses of VC, DLco and CPI also showed favourable changes with NAC. The effects of NAC on VC, DLco and CPI were significantly better if the baseline CPI was 50 points or lower. CONCLUSION: This descriptive analysis confirms and extends the favourable effects of NAC on lung function in IPF and emphasizes the usefulness of VC, DLco, and the CPI for the evaluation of a therapeutic effect. Most importantly, less progressed disease as indicated by a CPI of 50 points or lower at baseline was more responsive to therapy in this study.
Source: Respiratory Research
Authors: the Ifigenia study group*, Jürgen Behr, Maurits Demedts, Roland Buhl, Ulrich Costabel, Richard PN Dekhuijzen, Henk M Jansen, William MacNee, Michiel Thomeer, Benoit Wallaert, Francois Laurent, Andrew G Nicholson, Eric K Verbeken, Johny Verschakelen, CDR Flower, Stefano Petruzzelli, Paul De Vuyst, van den JMM Bosch, Eulogio Rodriguez-Becerra, Ida Lankhorst, Marco Sardina, Gabrielle Boissard
Institutions: LMU Klinikum, Ludwig-Maximilians-Universität München, KU Leuven, Johannes Gutenberg University Mainz, University Medical Center of the Johannes Gutenberg University Mainz, Ruhrlandklinik, University of Duisburg-Essen, Amsterdam UMC Location University of Amsterdam, University of Edinburgh, Centre Hospitalier Universitaire de Lille, Hôpital Albert Calmette, Centre Hospitalier Universitaire de Bordeaux, Royal Brompton Hospital, University of Pisa, Université Libre de Bruxelles, Erasmus Hospital, St. Antonius Ziekenhuis, Hospital Universitario Virgen del Rocío, Zambon (Italy)
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $1,255
OpenAlex ID: W2131944618